Here we report on a 51-year-old man with the primary diagnosis of cholangiocarcinoma. Workup with CT and contrast-enhanced ultrasound revealed an additional lesion in the spleen, raising the concern for metastasis. Combined FDG PET/CT revealed a different metabolic pattern, making a metastasis unlikely. Histopathology of the splenic lesion confirmed sclerosing angiomatoid nodular transformation, a rare benign lesion of the spleen.